Disease assessment in Waldenstrom Macroglobulinemia (WM) is dependent on the percent involvement of B-cell neoplasm in the bone marrow and IgM paraprotein in the serum. A subset of patients also demonstrates extramedullary involvement, which is infrequently examined. The role of extramedullary involvement in the diagnosis and prognosis of WM is poorly understood. The purpose of this study is to report the characteristics of WM patients with extramedullary disease (EMD). Nine hundred and eight-five patients with WM were evaluated at one academic center and the presence of EMD was assessed in these patients. Forty-three (4.4%) patients were identified to have EMD. Nine (21%) patients presented with involvement at WM diagnosis, while 34 (79%) developed EMD post-therapy for WM. Most frequent EMD sites involved were pulmonary (30%), soft tissue (21%), cerebrospinal fluid (23%), renal (8%), and bone (9%). The median overall survival at 10 years was 79% (95% CI: 57-90%). This is the first study to describe the clinical characteristics, response and overall survival in patients with extramedullary WM. Further studies to define the molecular characteristics of this entity and mechanisms of its development are warranted.
Background Sweden is home to a large and growing population of refugee youths who may be at risk of mental health problems such as post-traumatic stress disorder (PTSD). Thus, there is a need for interventions that address mental health problems in these populations. Schools have been identified as an ideal setting for delivering such interventions as they offer a non-stigmatizing space and are often central to young refugees’ social networks. The RefugeesWellSchool trial in Sweden will investigate an intervention comprising two programmes: Teaching Recovery Techniques (TRT) and In-service Teacher Training (INSETT), delivered in a school setting, among refugee youth. TRT is a group-based programme for children and adolescents, informed by Trauma-Focused Cognitive Behavioral Therapy (TF-CBT). INSETT is a multi-module course for teachers providing information on trauma and the refugee experience to build teachers’ cultural competence and capacity for supporting refugee youths in schools. Methods This trial employs a cluster randomized-control design with two arms: (1) the intervention arm in which the TRT and INSETT programmes are offered (n = 350), (2) the wait-list control arm (n = 350) in which services are provided as usual until the TRT and INSETT programmes are offered approximately six months later. Data will be collected prior to the intervention, immediately following the intervention, and at three months post-intervention. Outcomes for the trial arms will be compared using linear mixed models or ANCOVA repeated measures as well as the Reliable Change Index (RCI). Discussion This study will provide knowledge about the effectiveness of an intervention comprising two programmes: a group-based programme for youth reporting symptoms of PTSD and a training course for teachers, in order to build their competence and ability to support refugee youths in schools. Trial registration ISRCTN, ISRCTN48178969, Retrospectively registered 20/12/2019.
Purpose Resettled refugee youths are increasingly entering host-country school systems and are at risk of poor mental wellbeing. Schools and teachers are often expected to provide psychosocial support to youths with refugee backgrounds, but the teachers’ views on this expectation are poorly understood. We investigated the question: What do Swedish teachers believe is the role of (1) schools and (2) teachers in supporting refugee youths’ mental wellbeing? Method Four semi-structured focus groups were conducted with 30 Swedish educators from five schools. Interview transcripts were analysed and themes reflecting the key concepts were constructed using reflexive thematic analysis. Results Seven themes were constructed. Three addressed the role of schools in supporting refugee youths’ mental wellbeing: Promoting Belonging, Offering Refuge, and Instilling Civic Literacy. Four addressed the role of teachers: Building Relationships with Students, Maintaining a Non-therapeutic Relationship, Connecting to Professionals, and Instructing in the Classroom. Conclusion Teachers believe that both schools and teachers play an important role in supporting refugee youths’ mental wellbeing and each contributes in unique ways. However, schools and teachers are not always successful in supporting refugee youth and teachers reported facing challenges such as unclear roles and a lack of resources.
Introduction Disease assessment in Waldenstrom’s Macroglobulinemia (WM) is dependent on percent involvement of B-cell neoplasm in bone marrow and immunoglobulin M (IgM) paraprotein in the serum. A subset of patients also demonstrate extramedullary involvement and is infrequently examined. Its role in diagnosis and prognosis of WM is poorly understood. In this study, we sought to understand the role of extramedullary disease (EMD) in patients with WM. Methods Database for patients seen at Dana-Farber Cancer Institute was searched and 985 documented WM cases were identified between June 1994 and April 2013. Medical charts were reviewed for patients who had biopsy of one or more extramedullary sites during the course of disease. Involvement of lymph nodes, spleen and amyloid deposits were excluded. Results Among the 985 WM patients screened, 50 (5%) had evidence of extramedullary involvement during the course of disease. 28 (56%) were male and 22 (44%) were female, with median age of 57 years (min 37.8; max 71.1) at the time of diagnosis. At diagnosis, 11 (22%) patients were asymptomatic with monoclonal gammopathy of unknown significance (MGUS) or smoldering WM, 21 (42%) were low risk, 15 (30%) were intermediate risk, and 3 (6%) were high risk based on the Morel ISS-WM study (ISS-WM). Laboratory data included median beta-2 microglobulin of 3.7 g/dL (min 1.26; max 10.9), IgM of 3.3 g/dL (min 0.4; max 10.1), and percent bone marrow involvement of 37% (min 5%; max 90%). At the time of data collection, one patient was previously untreated, 14 (28%) had 1 line of therapy, 9 (18%) had 2 lines, 9 (18%) had 3 lines, 8 (16%) had 4 lines, and 9 (18%) had 5 or more lines of therapy. Among the 50 patients identified with EMD, 12 (24%) patients presented with involvement at diagnosis, while 38 (76%) developed EMD after receiving therapy. One patient had EMD at diagnosis, which continued post-diagnosis. 7 patients were identified with multiple sites of involvement during the course of disease. Overall, 60 extramedullary samples were noted with 47 (78%) occurring post-therapy. Extramedullary sites involved included: Pulmonary (n=14; 23%), Soft tissue (n=12; 20%), Cerebrospinal fluid (n=10; 17%), Renal (n=5; 8%), Bone (n=4; 7%), Peripheral blood (n=3; 5%), Neck mass (n=3; 5%), Skin (n=2; 3%), Breast (n=1; 2%), Conjunctiva (n=1; 2%), Liver (n=1; 2%), Gallbladder (n=1; 2%), Small Bowel (n=1; 2%), Prostate (n=1; 2%), and Colon (n=1; 2%). Immunophenotypic data was obtained for 58 of the 60 extramedullary samples and was analyzed by flow cytometry (n=24), immunohistochemistry (n=17), or both (n=17). All samples were of B-cell lineage (CD19+, CD20+). Monotypic surface immunoglobulin kappa or lambda light chain was analyzed in 49 samples, of which 43 were kappa (88%) and 6 were lambda (12%). In the group of 38 patients that presented with EMD post-therapy median time to EMD presentation from diagnosis was 75.7 months (min 1.5; max 213.7 or approximately 6 years) with a median of 2 lines of treatment (min 1; max 6). Therapies prior to EMD presentation in this group included rituximab based regimen in 31 of 38 cases (82%), cyclophosphamide based regimen in 15 cases (39%), fludarabine/cladribine based regimen in 12 cases (32%), and bortezomib based regimen in 11 cases (29%). Treatments for EMD in both groups included bendamustine based regimen resulting in minimal response (MR) or better in 8 of 9 cases (89%), rituximab based regimen resulting in MR or better in 24 of 30 cases (80%), cyclophosphamide based regimen resulting in MR or better in 11 of 14 cases (79%), and bortezomib based regimen resulting in MR or better in 6 of 8 cases (75%). At the time of data collection, 32 of 50 patients were alive (64%), 10 patients were lost to follow-up (20%), and 8 patients died due to progressive disease (16%). Conclusion This is the first description and analysis of the EMD in WM as a clinical entity and further studies in understanding the molecular mechanisms that govern EMD in WM should be examined. Disclosures: Treon: Millennium: Consultancy. Ghobrial:Onyx: Membership on an entity’s Board of Directors or advisory committees; BMS: Membership on an entity’s Board of Directors or advisory committees; BMS: Research Funding; Sanofi: Research Funding; Novartis: Membership on an entity’s Board of Directors or advisory committees.
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